acid-phosphatase and Myelodysplastic-Syndromes

The case history of a 70-year-old man with myelodysplastic syndrome terminated into acute leukemia in 22 months is presented. The leukemic cells exhibited multifocal acid phosphatase positivity and expressed TdT, CD45, CD34 and HLA-DR but not myeloid, monocytic or megakaryocytic differentiation antigenes. The genotypic analysis revealed clonal immunoglobulin heavy chain gene rearrangement. These phenotypic and genotypic analyses of the blastic cell population suggest that myelodysplastic syndrome may transform to pure acute lymphoblastic leukemia of B-cell origin.

Topics: Acid Phosphatase; Aged; Amino Acid Sequence; Antigens, CD34; B-Lymphocytes; Base Sequence; Cell Differentiation; Cell Transformation, Neoplastic; Gene Rearrangement, B-Lymphocyte; Genes, Immunoglobulin; Humans; Immunophenotyping; Leukemia, B-Cell; Leukocyte Common Antigens; Male; Molecular Sequence Data; Myelodysplastic Syndromes

Plasmacytic morphologic characteristics are usually associated with cells of B-lymphocyte origin. Recently, plasmacytoid T-cells have been described in reactive lymph nodes and a rare form of lymphoma characteristically associated with myeloproliferative disorders. This report documents a case of plasmacytoid T-cell malignancy that initially presented as an acute leukemia in an elderly man with a longstanding myelodysplastic syndrome. The tumor replaced bone marrow and involved lymph nodes. Despite aggressive therapy, he died quickly of his leukemia/lymphoma. This case illustrates the need for complete cellular analysis in the diagnosis of morphologically plasmacytic malignancies and raises additional questions about the relationship of this peculiar type of T-cell to the hematopoietic marrow.

Topics: Acid Phosphatase; Aged; Antibodies, Monoclonal; Antigens, CD; Cell Cycle; Diagnosis, Differential; DNA, Neoplasm; Fluorescent Antibody Technique; Gene Rearrangement, beta-Chain T-Cell Antigen Receptor; Histocytochemistry; Humans; Karyotyping; Leukemia, Lymphocytic, Chronic, B-Cell; Leukemia, Plasma Cell; Male; Microscopy, Electron; Myelodysplastic Syndromes; T-Lymphocytes

Blood monocyte differentiation to macrophages was examined in nine patients with primary myelodysplastic syndromes using the skin window technique. Emigrated cells were stained cytochemically for acid phosphatase reaction after 1, 2, 4, 7, 9, 12 and 23 h. Compared to age-matched controls, seven patients showed a significant delay in lysosomal enzyme acquisition, which is associated with macrophage differentiation. Our results with this in-vivo assay demonstrate an involvement of the monocyte/macrophage system in primary myelodysplastic syndromes and show that patients often have a disturbance in macrophage differentiation.

Topics: Acid Phosphatase; Cell Differentiation; Humans; Macrophages; Myelodysplastic Syndromes; Skin